Werner syndrome


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Related to Werner syndrome: progeria, ataxia telangiectasia

Wer·ner syn·drome

(wĕr'nĕr), [MIM*277700]
a premature aging disorder presenting sclerodermalike skin changes, bilateral juvenile cataracts, progeria, hypogonadism, and diabetes mellitus; autosomal recessive inheritance, caused by mutation in the WRN gene, which encodes a helicase protein on chromosome 8p.

Wer·ner syn·drome

(wĕr'nĕr), [MIM*277700]
a premature aging disorder presenting sclerodermalike skin changes, bilateral juvenile cataracts, progeria, hypogonadism, and diabetes mellitus; autosomal recessive inheritance, caused by mutation in the WRN gene, which encodes a helicase protein on chromosome 8p.

Werner syndrome

(vĕr′nər) or

Werner's syndrome

(-nərz)
n.
An autosomal recessive disorder characterized by short stature and the appearance of premature aging after puberty, with early development of conditions such as cataracts, cardiovascular disease, osteoporosis, and malignancies.

Werner syndrome

A rare autosomal recessive progeroid syndrome (OMIM:277700) characterised by premature onset of multiple age-related disorders, including atherosclerosis, cancer, type-2 diabetes, ocular cataracts, osteoporosis, and death by age 47, often due to myocardial infarction.

Molecular pathology
Defects of WRN, which encodes a DNA helicase responsible for maintaining genomic integrity, cause Werner syndrome.

Wer·ner syn·drome

(ver'ner sin'drōm)
A premature aging disorder consisting of sclerodermalike skin changes, bilateral juvenile cataracts, progeria, hypogonadism, and diabetes mellitus; autosomal recessive inheritance, caused by mutation in the WRN gene, which encodes a helicase protein on chromosome 8p.

Werner,

Otto, German physician, 1879-1936.
Werner disease - hereditary disorder characterized by premature aging. Synonym(s): progeria adultorum; Werner syndrome
Werner syndrome - Synonym(s): Werner disease
References in periodicals archive ?
Goto, "A case of Werner syndrome with three primary lesions of malignant melanoma," Journal of Dermatology, vol.
"Our study connects the dots between Werner syndrome and heterochromatin disorganization, outlining a molecular mechanism by which a genetic mutation leads to a general disruption of cellular processes by disrupting epigenetic regulation," says Izpisua Belmonte.
Homozygous and compound heterozygous mutations at the Werner syndrome locus.
A case of a delusional psychotic syndrome in the setting of Werner syndrome. Br J Psych 1988; 152: 703-70.
A case of myocardial infarction in a patient with Werner syndrome. Texas Heart Institute J 1984; 11: 80-3.
Accelerated loss of telomeric repeats may not explain accelerated replicative decline of Werner syndrome cells.
Mean telomere Telomerase Human cell line/tissue length, kbp activity (a) Sperm (donor: 20 years) 12 + Sperm (donor: 68 years) +6 + Fetal kidney 20 - Fetal liver +7 Unknown Colon mucosa +0 - Colon cancer 3-4 + Skin tumor 4-10 + Breast tumor 4-10 + Lung tumor 4-10 + Kidney tumor 4-10 + Fetal fibroblasts in vitro 8.6 - Fibroblasts (71 years) in vitro 6.5 - Fetal fibroblasts in vivo 8.6 - Fibroblasts (93 years) in vivo 7.1 - Fibroblasts (9-14 years) 8.8 - Fibroblasts progeria patient (0-14 5.4 Unknown years) Fibroblasts Werner syndrome patient 4.7 Unknown (a) Present (1), absent (2).
Martin and a Japanese research group headed by Toshio Ogihara of the Osaka University Medical School have recently started to study two known normal versions of the Werner syndrome gene.
Schellenberg of the University of Washington in Seattle and his colleagues located the Werner syndrome gene on chromosome 8.
In the April 12 Science, the researchers describe four different mutations of the gene that they found in Werner syndrome patients.
For people with Werner syndrome, the discovery does not suggest an obvious treatment.